connective tissue dysplasia
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Author(s):  
М.С. Селихова ◽  
В.В. Скворцов ◽  
Г.В. Ершов ◽  
А.Г. Ершов ◽  
Г.И. Малякин

Одним из наиболее значимых факторов риска формирования опущений и выпадений внутренних половых органов являются заболевания соединительной ткани. Неуклонный рост числа женщин, страдающих пролапсом органов малого таза, привел к тому, что данная патология стала как медицинской, так и социально-экономической проблемой во многих странах. В то же время данные о роли недифференцированных дисплазий соединительной ткани в развитии пролапса органов малого таза и в формировании недостаточности тазового дна и возможности использования ее с целью прогнозирования данной патологии разноречивы. С целью определения взаимосвязи проявления недифференцированных дисплазий соединительной ткани с развитием пролапса органов малого таза был проведен ретроспективный анализ 157 историй болезни пациенток с пролапсом гениталий. У 27,39% пациенток были отмечены проявления дисплазий соединительной ткани. В 39,53% случаев было выявлено сочетание проявления недифференцированных дисплазий соединительной ткани с родовым травматизмом. У 16,28% от количества случаев с родовым травматизмом и пролапсом органов малого таза выявлены выраженные формы дисплазии соединительной ткани (варикозное расширение вен нижних конечностей, полипоз кишечника, пролапс митрального клапана), потребовавшие хирургической коррекция до беременности или после родов. Выявленная у каждой третьей пациентки, оперированной по поводу несостоятельности тазового дна, недифференцированная дисплазия соединительной ткани позволяет рассматривать ее как фактор риска развития пролапса органов малого таза, особенно в сочетании с травматизацией промежности в родах. Однако для оценки роли и значимости патологии соединительной ткани в прогнозировании формирования опущения и выпадения внутренних половых органов малого таза у женщин необходимы проспективные рандомизированные исследования. One of the most significant risk factors for the formation of internal genital organs is connective tissue diseases. The steady increase in the number of women suffering from pelvic organ prolapse has led to the fact that this pathology has become both a medical and socio-economic problem in many countries. At the same time, the data on the role of undifferentiated connective tissue dysplasias in the development of pelvic organ prolapse and in the formation of pelvic floor insufficiency and the possibility of using it to predict this pathology are contradictory. To determine the relationship between the manifestation of undifferentiated connective tissue dysplasia and the development of pelvic organ prolapse, a retrospective analysis of 157 case histories of patients with genital prolapse was carried out. In 27,39% of patients, manifestations of connective tissue dysplasia were noted. In 39,53% of cases, a combination of the manifestation of undifferentiated connective tissue dysplasias with birth traumatism was revealed. Severe forms of connective tissue dysplasia (varicose veins of the lower extremities, intestinal polyposis, mitral valve prolapse) were found in 16,28% of the number of cases with birth traumatism and pelvic organ prolapse, which required surgical correction before pregnancy or after childbirth. Revealed in every third patient operated on for pelvic floor incompetence, undifferentiated connective tissue dysplasias allows us to consider it as a risk factor for pelvic organ prolapse, especially in combination with perineal trauma during childbirth. However, to assess the role and significance of connective tissue pathology in predicting the formation of prolapse and prolapse of the internal genital organs of the small pelvis in a woman, prospective randomized studies are needed. Keywords: genital prolapse, connective tissue dysplasia, risk factors, social-active age, surgical treatment.


2021 ◽  
Vol 12 (4) ◽  
pp. 27-33
Author(s):  
I. A. Shevnin ◽  
N. A. Ilyushchenko ◽  
O. N. Ragozin ◽  
O. V. Ragozin ◽  
N. V. Ermakova

Background. Assessment of the physical development of children in individual regions of the Russian Federation does not lose its relevance and requires constant updating, in this regard, it is important to study it in children adapting to the climatic and geographical conditions of the North, as well as the effect of undifferentiated connective tissue dysplasia on it. Aim. Aim of the study is to study the gender and age characteristics of the proportionality of physical development in children with undifferentiated connective tissue dysplasia living in the Khanty-Mansiysk Autonomous Okrug Yugra. Materials and methods. The analysis of physical development of 528 children of the second childhood and adolescence, living in the Khanty-Mansi Autonomous Okrug Yugra, was carried out. The study group consisted of 342 children (248 boys and 94 girls) with a diagnostically significant number of undifferentiated connective tissue dysplasia symptoms (from 6 to 18 stigmas). The comparison group included 186 children (111 boys and 75 girls) who did not score a significant threshold of phene signs (from 0 to 5 stigmas). Physical development was assessed according to the Quetelet II weight-height index, chest-weight indices: Pignet, Vervek, and Brugsch, as well as proportionality indices: sternum, shoulder width, pelvic width, torso shape, arm and leg length indices, cranial and facial indices, interorbital-circular index. Results. Children without undifferentiated connective tissue dysplasia during the second childhood have a tendency to malnutrition, and are characterized by a weak physique with a tendency to narrow chest. In adolescence, the tendency towards weakness of the physique persists, while height-weight ratios deviate towards normotrophy. Children with undifferentiated connective tissue dysplasia during the second childhood and adolescence have a rectangular or trapezoidal body with an average length, as well as long upper and lower limbs relative to the length of the body. Conclusion. The impact of the socio-ecological factors of the North neutralizes the differences in the rates of physical development in healthy children and persons with disorders caused by congenital disorders of organogenesis of the undifferentiated connective tissue dysplasia type.


2021 ◽  
Author(s):  
I.A. Shevnin ◽  
O.N. Ragozin ◽  
O.V. Ragozina ◽  
N.A. Ilyushchenko ◽  
B.Z. Jafarova

Undifferentiated forms of DST are widespread among children living in Khanty-Mansiysk, in connection with which it is relevant to assess the influence of this pathology on the formation of posture in schoolchildren of the North. Determination of the type of posture and the identification of stigmas of dysembryogenesis was carried out in 208 schoolchildren of the city of Khanty-Mansiysk. Posture types were divided into: normal, round, flat and stooped. Participants with the number of stigmas from 0 to 5 were allocated to the comparison group (n = 66), the rest (6 or more) made up the study group (n = 143). A card was entered, including 43 hair dryers. The study found that the incidence of UCTD among children in the Khanty-Mansi Autonomous Okrug-Yugra is noticeably higher than among those living in temperate latitudes and has a negative effect on the formation of posture. Key words: North, posture, physical development, undifferentiated connective tissue dysplasia.


2021 ◽  
Author(s):  
N.A. Ilyushenko ◽  
Yu.O. Gainutdinova ◽  
Z.B. Eskindyrova ◽  
O.V. Ragozina ◽  
I.A. Shevnin ◽  
...  

The article analyzes the data of an anthropometric and somatotypological study of 164 boys and 93 girls of the northern region, different sexual somatotypes with different severity of signs-phenes of undifferentiated connective tissue dysplasia. It was found that 44% of boys and 22% of girls with d, according to sexual somatic differentiation, correspond to the biological sex, 34% of boys and 53% of girls have mild gender dysplasia in the form of mesomorphy, and 19% of boys and 21% of girls have signs of sex inversion. Undifferentiated connective tissue dysplasia affects the severity of morphotypological differences between sexual somatotypes and the appearance of signs of gracialization within them. Key words: northern region, adolescence, sexual dimorphism, undifferentiated connective tissue dysplasia.


2021 ◽  
Vol 20 (7) ◽  
pp. 2973
Author(s):  
Ya. B. Khovaeva ◽  
A. L. Korovin ◽  
Ya. A. Sychugov ◽  
L. V. Ermachkova ◽  
N. P. Moiseenko

The article presents a case report of spontaneous coronary artery dissection (SCAD) in the young woman with signs of undifferentiated connective tissue dysplasia (UCTD). The patient was admitted urgently with a clinical and electrocardiographic signs of acute ST-segment elevation coronary syndrome. The patient underwent a coronary angiography, which revealed a type D linear intimal dissection. The decision was made to perform balloon vasodilation with the placement of a drug-eluting stent. With multicomponent therapy, the patient’s condition improved. Physical examination revealed external markers of UCTD. Therefore, it was quantified by systems and organs. The patient had no other cardiovascular diseases, previous infection, or other trigger factors. She was discharged with recommendations to continue the prescribed therapy. This case report demonstrates SCAD as a rare cause of acute myocardial infarction in a young patient. The background for SCAD development in this case was UCTD. Algorithms for managing patients with SCAD against the background of UCTD have not been defined and require further study.


2021 ◽  
pp. 91-98
Author(s):  
A. A. Yusufov ◽  
I. A. Cherkasova ◽  
E. A. Lavrova ◽  
A. A. Plyukhin

To study the cardiac and visceral signs of connective tissue dysplasia in children with juvenile rheumatoid arthritis, a comprehensive ultrasound examination of the abdominal cavity organs and echocardiography examination were conducted according to the standard protocol in 52 children aged from 5 to 16 years with the diagnosis of juvenile rheumatoid arthritis. The reference group consisted of 26 patients with autonomic dysfunction (vegetative-vascular dystonia). The incidence of such visceral dysplasia types as duodenogastric reflux, gastroesophageal reflux, and gallbladder malformations among children with juvenile rheumatoid arthritis was shown higher than in the reference group. The main cardiac sign of connective tissue dysplasia in patients with juvenile rheumatoid arthritis are an excessive chord of the left ventricle.


2021 ◽  
pp. 25-31
Author(s):  
O.Yu. Chumak ◽  
◽  
A.P. Volokha ◽  
◽  

Undifferentiated connective tissue dysplasia (UCTD) in pregnant women reduces the adaptive capacity of their newborns and is an unfavorable background for the development of certain pathological conditions of the perinatal period. At the same time, information on the spectrum of diseases in children born to mothers with clinical manifestations of UCTD is rather contradictory. Purpose — to study the nature and direction of the correlation between certain pathological conditions in newborns and clinical signs of UCTD in their mothers. Materials and methods. We examined 75 women in labor aged 16 to 44 years old and their 75 newborn children (38 boys and 37 girls) during 2018–2020. The number of girls and boys is approximately the same, the gender difference between children did not affect on the results of the study. The newborns gestational age (GA) was 28–42 weeks, and their birth weight (BW) was 1500–4070 g. Among them there were 51 (68%) full-term, and 24 (32%) — preterm infants. Anthropometric indices (AI), which were used to confirm dolichostenomelia as a UCTD marker, were calculated for mothers and their children. The integral indicator of dolichostenomelia (IID) was determined by summing the standardized values of these coefficients for each mother and child. The presence of neonatal encephalopathy, congenital pneumonia, interventricular septal defect (IVSD), intrauterine growth retardation (IUGR), respiratory distress syndrome, asphyxia at birth, grade I–II of intraventricular hemorrhage (IVH), and necrotizing enterocolitis were taken into account in children. As for mothers, the presence of complications of their pregnancy and labor associated with UCTD was taken into consideration, and in addition, hypermobility of the joints was assessed according to the Beighton scale. Women were divided into two groups: group I (n=45) consisted of women with >1 anthropometric marker of UCTD, group II (n=30) involved mothers who did not have any of these markers. Newborn children were divided into two groups, respectively: group I involved newborns from mothers with signs of UCTD, group II consisted of borned from mothers without signs of UCTD. Results. It was found that mothers with UCTD markers reliably more often had children weighing less than 2500 g (ϕс=0.251; р=0.029). According to the results of the morbidity analysis, it was clarified that children borned from mothers with UCTD manifestations, were more often suffering from congenital pneumonia (ϕс=0.218; р=0.049), IVSD (ϕс=0.241; р=0.037) and IUGR (ϕс=0.31; р=0.029). According to the results of the rank correlation analysis, a direct moderate correlation was established between the presence of progression of varicose veins in women during pregnancy and IID both in them (ρ=0.463; p<0.001) and in their children (ρ=0.369; p=0.001); a similar in degree and direction correlation of >3 UCTD associated obstetric complications with IID of mothers (ρ=0.305; p=0.008) and their children (ρ=0.326; p=0.004) was also found. At the same time, a positive weak correlation was established between mothers' IID and registration of placental dysfunction (ρ=0.231; p=0.046), polyhydramnios (ρ=0.234; p=0.043) in them. As for newborns, their IID had a direct moderate correlation with their mothers' IPD (ρ=0.364; p=0.001), and a weaker correlation with joint hypermobility in their mothers (ρ=0.258; p=0.025) and obstetric ruptures (ρ=0.230; p=0.047). Simultaneously, there were no statistically reliable differences between the groups of children by gender, their GA, age of their mothers and the mode of delivery. Conclusions. Consequently, children borned from mothers with clinical markers of UCTD are more often have low BW (p=0.029); during the neonatal period they have IUGR (p=0.029), congenital pneumonia (p=0.049) and IVSD (p=0.037) more often registered. It should also be noted that integral anthropometric markers of UCTD in newborn children have the most significant correlation with similar markers in their mothers (p=0.001), on the one hand, as well as with the progression of varicose disease in women during pregnancy (p=0.001) and a combination >3 UCTD associated obstetric complications (p=0.004), on the other hand. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: newborns, postpartum women, undifferentiated connective tissue dysplasia.


2021 ◽  
Vol 4 (2) ◽  
Author(s):  
Iryna Romash

When the structure of the connective tissue is disturbed, the probability of developing pathology of the internal organs increases. The gastrointestinal tract is one of the systems that is most often involved in the pathological process in the case of such disorders. Due to its mesenchymal origin, the esophagus is one of the first to suffer. Normalization of mineral metabolism, correction of immunological and bioenergetic status are the main tasks in the treatment of such patients. Therapy with magnesium drugs, vitamins, anabolic drugs, ?-blockers, aldosterone antagonists, and amino acid drugs should also be used for this purpose. Patients in this group should also take drugs that contain vitamin D3


Author(s):  
A.N. Proshin ◽  
V.V. Mashin ◽  
L.A. Belova ◽  
D.V. Belov

The aim of the study is to examine the characteristics of intracranial veins (magnetic resonance angiography), and the elastic-viscous properties of the red blood cell membranes (atomic force microscopy) in adolescents with connective tissue dysplasia. Material and Methods. The authors examined 156 adolescents. The main group consisted of 82 adolescents with connective tissue dysplasia. The comparison group included 74 adolescents without connective tissue dysplasia. Connective tissue dysplasia was assessed according to L.N. Abakumova scale (2006). Magnetic resonance angiography was performed on a Siemens Magnetom Symphony apparatus equipped with a superconducting magnetic system with 1.5 Tesla field strength. Preparations of dry red blood cells were made to study red blood cell cytoplasmic membrane. The specimen was scanned using an atomic force microscope SOLVER P47-Pro. Results. The majority of adolescents from the main group demonstrated anatomical variants of the dura mater sinuses structure. At the same time, sinus hypoplasia in patients of the main group was diagnosed more often than asymmetry of these structures. In the comparison group, the number of anatomical variants of the dura mater sinuses structure also prevailed over the symmetrical structure. However, the sinuses asymmetry was more often. Comparative analysis of Young's modulus values in two groups of patients showed a statistically significant superiority of this indicator in the main group. Correlation analysis of the connective tissue dysplasia severity and Young's modulus values revealed a moderate direct correlation. Conclusion. Thus, it can be assumed that in patients with connective tissue dysplasia the cerebral vessel structure and elastic properties of red blood cell membranes formed during embryogenesis impair the functional and adaptive capabilities of intracranial vessels, contributing to the development and progression of cerebral venous disorders and their subsequent clinical manifestation. Key words: intracranial veins, Young's modulus, connective tissue dysplasia. Цель. Исследовать при помощи магнитно-резонансной ангиографии особенности интракраниальных вен и методом атомно-силовой микроскопии – упруго-вязкостные свойства мембран эритроцитов подростков с соединительнотканной дисплазией. Материалы и методы. Обследовано 156 подростков. Основную группу (ОГ) составили 82 подростка с признаками дисплазии соединительной ткани (ДСТ), группу сравнения (ГС) – 74 подростка без проявлений ДСТ. ДСТ оценивали по шкале Л.Н. Абакумовой (2006). Магнитно-резонансная ангиография проводилась на аппарате Siemens Magnetom Symphony, оснащенном сверхпроводящей магнитной системой с силой поля 1,5 Тесла. С целью изучения состояния цитоплазматической мембраны эритроцитов были изготовлены сухие препараты эритроцитов. Препарат подвергался сканированию, которое проводилось с помощью атомно-силового микроскопа SOLVER P47-Pro. Результаты. У большинства подростков ОГ выявлены анатомические варианты строения синусов твердой мозговой оболочки. При этом гипоплазия синусов у пациентов ОГ диагностировалась чаще, чем асимметрия данных структур. В ГС количество анатомических вариантов строения синусов твердой мозговой оболочки также превалировало над симметричным строением, но чаще диагностировалась асимметрия синусов. Сравнительный анализ значений модуля Юнга в двух группах пациентов показал статистически значимое превосходство данного показателя в ОГ. Корреляционный анализ степени выраженности ДСТ и значений модуля Юнга выявил умеренную прямую корреляцию. Выводы. Можно предположить, что имеющиеся при ДСТ особенности структуры церебральных сосудов и эластических свойств мембран эритроцитов, заложенные в процессе эмбриогенеза, ухудшают функциональные и адаптационные возможности интракраниальных сосудов, что способствует возникновению и прогрессированию церебральных венозных расстройств и их последующей клинической манифестации. Ключевые слова: интракраниальные вены, модуль Юнга, дисплазия соединительной ткани.


2021 ◽  
Vol 9 (2) ◽  
pp. 10-13
Author(s):  
T.V. Frolova

Background. In practice, patients with numerous undifferentiated forms of connective tissue dysplasia are more common. With regard to the combination of myopia and undifferentiated connective tissue dysplasia, one of the links in the mechanism of origin of this refractive anomaly is decreased thickness of the sclera and a change in its elastic parameters. Myopia in children often occurs against the background of impaired blood supply to the visual analyzer associated with circulatory pathology in the vertebral vessels. The purpose was to evaluate cerebral blood flow in patients with myopia in combination with undifferentiated connective tissue dysplasia. Materials and methods. The study involved 86 children (170 eyes) diagnosed with progressive myopia. All patients were consulted by related specialists to detect undifferentiated connective tissue dysplasia and underwent a complete ophthalmological exa-mination. The diagnostic coefficient of expression of phenotypic and clinical signs of undifferentiated connective tissue dysplasia was also determined using the expert table of T. Kadurina. Results. Phenotypic and clinical signs of undifferentiated connective tissue dysplasia were noted in 41 (100 %) cases when evaluating according to the criteria of T. Mil-kovskaya-Dimitrova and T. Kadurina, and in 34 (83.0 %) children du-ring screening for joint hypermobility on Beighton score. As for increased axial length of the eye in the examined patients with myopia and undifferentiated connective tissue dysplasia, it was noted that an increase had a direct correlation with the severity of undifferentiated connective tissue dysplasia, with the greatest correlation obtained in children with pronounced phenotypic manifestations in the musculoskeletal system (correlation coefficient was 0.83). Chemical regulation of carotid artery tone (respiratory failure test) is altered in the vast majority of patients with myopia. Conclusions. All children with progressive myopia are re-commended to be examined by related specialists to identify undif-ferentiated connective tissue dysplasia and hemodynamic disorders. Only a comprehensive approach to this refractive anomaly can be effective in combating its progression.


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